Journal ArticleDOI
Muscle assembly: a titanic achievement?
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TLDR
Tin may serve as a molecular blueprint for sarcomere assembly and turnover by specifying the precise position of its ligands within each half-sarcomere in addition to functioning as a Molecular spring that maintains the structural integrity of the contracting myofibrils.About:
This article is published in Current Opinion in Cell Biology.The article was published on 1999-02-01. It has received 336 citations till now. The article focuses on the topics: Obscurin & Titin.read more
Citations
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The Giant Protein Titin: A Major Player in Myocardial Mechanics, Signaling, and Disease
Henk Granzier,Siegfried Labeit +1 more
TL;DR: In this paper, the authors focus on recent findings vis-a-vis titin's molecular spring segments in cardiac titins and discuss new insights regarding the role of titin as a biomechanical sensor and signaling molecule.
Journal ArticleDOI
The Complete Gene Sequence of Titin, Expression of an Unusual ≈700-kDa Titin Isoform, and Its Interaction With Obscurin Identify a Novel Z-Line to I-Band Linking System
Marie Louise Bang,Thomas Centner,Friderike Fornoff,Adam J. Geach,Michael Gotthardt,Mark McNabb,Christian Witt,Dietmar Labeit,Carol C. Gregorio,Henk Granzier,Siegfried Labeit +10 more
TL;DR: The complete sequence of the human titin gene contains 363 exons, which together code for 38 138 residues (4200 kDa), and in its central I-band region, 47 novel PEVK exons were found, which contribute to titin’s extensible spring properties.
Journal ArticleDOI
Mutations of TTN, encoding the giant muscle filament titin, cause familial dilated cardiomyopathy.
Brenda Gerull,Michael Gramlich,John Atherton,Mark McNabb,Karoly Trombitás,Sabine Sasse-Klaassen,Jonathan G. Seidman,Christine E. Seidman,Henk Granzier,Siegfried Labeit,Michael P. Frenneaux,Ludwig Thierfelder,Ludwig Thierfelder +12 more
TL;DR: It is shown that mutations in the gene encoding giant-muscle filament titin (TTN) cause autosomal dominant DCM linked to chromosome 2q31 (CMD1G; MIM 604145), and the identification of TTN mutations in individuals with C MD1G should provide further insights into the pathogenesis of familial forms of CHF and myofibrillar titin turnover.
Journal ArticleDOI
Reverse engineering of the giant muscle protein titin
Hongbin Li,Wolfgang A. Linke,Andres F. Oberhauser,Mariano Carrión-Vázquez,Jason Kerkvliet,Hui Lu,Piotr E. Marszalek,Julio M. Fernandez +7 more
TL;DR: This work uses protein engineering and single-molecule atomic force microscopy to examine the mechanical components that form the elastic region of human cardiac titin and shows the functional reconstitution of a protein from the sum of its parts.
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Molecular basis of muscular dystrophies.
Ronald D. Cohn,Kevin P. Campbell +1 more
TL;DR: A large number of genes involved in muscular dystrophy encode components of the dystrophin‐glycoprotein complex (DGC) which normally links the intracellular cytoskeleton to the extracellular matrix, which is thought to lead to loss of sarcolemmal integrity and render muscle fibers more susceptible to damage.
References
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Journal ArticleDOI
Reversible Unfolding of Individual Titin Immunoglobulin Domains by AFM
TL;DR: Single-molecule atomic force microscopy was used to investigate the mechanical properties of titin, the giant sarcomeric protein of striated muscle, and refolding of immunoglobulin domains was observed.
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Molecular diversity of myofibrillar proteins: gene regulation and functional significance
TL;DR: The pattern of isogene expression varies during muscle development in relation to the different origin of myogenic cells and primary/secondary fiber generations and is affected by neural and hormonal influences.
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Folding-Unfolding Transitions in Single Titin Molecules Characterized with Laser Tweezers
TL;DR: Scaling the molecular data up to sarcomeric dimensions reproduced many features of the passive force versus extension curve of muscle fibers, including force hysteresis arises from a difference between the unfolding and refolding kinetics of the molecule relative to the stretch and release rates in the experiments.
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Titins: Giant Proteins in Charge of Muscle Ultrastructure and Elasticity
TL;DR: The architecture of sequences in the A band region of titin suggests why thick filament structure is conserved among vertebrates and compares two elements that correlate with tissue stiffness that suggest that titin may act as two springs in series.
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Elasticity and unfolding of single molecules of the giant muscle protein titin.
TL;DR: Mechanical experiments on single molecules of titin are done to determine their visco-elastic properties, showing that there are two main sources of elasticity: one deriving from the entropy of straightening the molecule; the other consistent with extension of the polypeptide chain in the PEVK region.